Disorders of interstitial cells of Cajal
Disorders of interstitial cells of Cajal
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DOI：
10.1097/mpg.0b013e31812e65e0
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发表时间：
2007-12-01
影响因子：
2.9
通讯作者：
Burns, Alan J.
中科院分区：
文献类型：
--
作者：
Burns, Alan J.
在过去的20年里，间质细胞（ICCs）被认为是调节胃肠运动的重要因素。具体来说，它们已被证明对调节胃肠道平滑肌的相性收缩活动的电慢波的产生和传播至关重要，并介导从肠运动神经元到平滑肌细胞的神经传递。这些不同的功能作用是由不同表型的ICC进行的，这些ICC在肌膜内具有离散分布。通过研究缺乏ICC的突变小鼠，确定ICC在肠道中的功能作用已经得到了促进，这要么是由于酪氨酸激酶受体Kit的缺失，要么是其配体干细胞因子的缺失，这两者都是ICC正常发育所必需的。在人类中，在某些病理生理条件下，ICC网络的丢失或缺陷似乎在某些运动障碍的产生中起作用。在贲门失弛缓症、慢性肠假性梗阻、先天性巨结肠病、炎症性肠病和慢传导性便秘等情况下，有报道称ICC分布发生改变。分子和遗传技术正在帮助研究人员确定ICC网络的缺陷是运动障碍的原因，还是被破坏的ICC网络是肠道功能障碍的结果。
Interstitial cells of Cajal (ICCs) have, in the past 2 decades, been recognised as important elements in the regulation of gastrointestinal motility. Specifically, they have been shown to be critical for the generation and propagation of electrical slow waves that regulate the phasic contractile activity of gastrointestinal smooth muscle, and for mediating neurotransmission from enteric motor neurons to smooth muscle cells. These different functional roles are carried out by different phenotypic classes of ICC that have discrete distributions within the tunica muscularis. Identifying the functional roles of ICC within the gut has been facilitated by studying mutant mice deficient in ICC, either as a consequence of loss of the tyrosine kinase receptor, Kit, or its ligand, stem cell factor, both of which are necessary for normal ICC development. In humans, under certain pathophysiological conditions, loss or defects in ICC networks appear to play a role in the generation of certain motility disorders. Alterations in ICC distribution have been reported in conditions such as achalasia, chronic intestinal pseudoobstruction, Hirschsprung disease, inflammatory bowel diseases, and slow transit constipation. Molecular and genetic techniques are helping researchers to determine whether defects in ICC networks are the cause of motility disorders, or whether the disrupted ICC networks are a consequence of gut dysfunction.